Treatment and Outcome of Stiff-Man Syndrome

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چکیده

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Jerking stiff-man syndrome.

A female patient had permanent axial muscular rigidity similar to the "stiff-man syndrome", together with axial myoclonus triggered by stretch reflexes and by supramaximal stimulation of the supraorbital nerve. The disorder responded to treatment with diazepam and baclofen. This disorder bore a marked similarity to the so-called "jerking stiff-man syndrome".

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The stiff man syndrome (SMS) and its variants, focal SMS, stiff limb (or leg) syndrome (SLS), jerking SMS, and progressive encephalomyelitis with rigidity and myoclonus (PERM), appear to occur more frequently than hitherto thought. A characteristic ensemble of symptoms and signs allows a tentative clinical diagnosis. Supportive ancillary findings include (1) the demonstration of continuous musc...

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The 'stiff man syndrome' in a boy.

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ژورنال

عنوان ژورنال: Pediatric Neurology Briefs

سال: 2012

ISSN: 2166-6482,1043-3155

DOI: 10.15844/pedneurbriefs-26-3-7